Desmoid tumor risk factors

Desmoid tumor Microchapters
Home
Patient Information
Overview
Historical Perspective
Classification
Pathophysiology
Causes
Differentiating Desmoid tumor from other Diseases
Epidemiology and Demographics
Risk Factors
Natural History, Complications and Prognosis
Diagnosis
History and Symptoms
Physical Examination
Laboratory Findings
CT
MRI
Other Imaging Findings
Other Diagnostic Studies
Treatment
Medical Therapy
Surgery
Cost-Effectiveness of Therapy
Future or Investigational Therapies
Case Studies
Case #1

Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1] Associate Editor(s)-in-Chief: Sara Mohsin, M.D.[2]Faizan Sheraz, M.D. [3]

Overview

Common risk factors for the development of desmoid tumor include an APC mutation 3' to codon 1399, previous abdominal surgery, and the female sex.

Risk Factors

Common risk factors for the development of desmoid tumor include:

Common risk factors for development of desmoid tumor

Risk factors	Associated features
Familial adenomatous polyposis (FAP) (10-20%)	852 times elevated risk of developing desmoids in FAP patients as compared to general population Mostly desmoids in FAP are intra-abdominal or involve abdominal wall Most of these desmoids are unresectable associated with diffuse infiltration of mesentery FAP is associated with mutations in the APC (adenomatous polyposis coli) gene, located on chromosome 5q21-q22 Sometimes, only manifestation of APC mutation is desmoid FAP screening recommended in patients with multiple intraabdominal or truncal desmoid tumors Risk of finding FAP in patients who develop a desmoid without a prior history of FAP is relatively low (4.8% only)
Gardner syndrome
Specific location of APC (adenomatous polyposis coli) gene mutation i.e. 3' of codon 1444
Family history of desmoid tumor
Pregnancy	Extraabdominal and abdominal desmoids occur in women during or following pregnancy Presents as an abdominal mass separate from uterus Occurs in pregnancy due to: Pregnancy-associated high estrogen levels Trauma related to the pregnancy (including a scar from a prior Cesarean section)
History of antecedent trauma at the tumor site (30%)	Previous abdominal surgery in patients with FAP In one-half patients, desmoids can develop within first five years post-surgery
Female gender

• APC mutation 3' to codon 1399